Cribriform neuroepithelial tumor (CRINET): a nonrhabdoid ventricular tumor with INI1 loss and relatively favorable prognosis.
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Cribriform neuroepithelial tumor (CRINET): a nonrhabdoid ventricular tumor with INI1 loss and relatively favorable prognosis. / Hasselblatt, Martin; Oyen, Florian; Gesk, Stefan; Kordes, Uwe; Brigitte, Wrede; Bergmann, Markus; Schmid, Hansjörg; Frühwald, Michael C; Schneppenheim, Reinhard; Siebert, Reiner; Paulus, Werner.
In: J NEUROPATH EXP NEUR, Vol. 68, No. 12, 12, 12.2009, p. 1249-1255.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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T1 - Cribriform neuroepithelial tumor (CRINET): a nonrhabdoid ventricular tumor with INI1 loss and relatively favorable prognosis.
AU - Hasselblatt, Martin
AU - Oyen, Florian
AU - Gesk, Stefan
AU - Kordes, Uwe
AU - Brigitte, Wrede
AU - Bergmann, Markus
AU - Schmid, Hansjörg
AU - Frühwald, Michael C
AU - Schneppenheim, Reinhard
AU - Siebert, Reiner
AU - Paulus, Werner
PY - 2009/12
Y1 - 2009/12
N2 - Atypical teratoid/rhabdoid tumors are malignant embryonal tumors characterized by the presence of rhabdoid cells, genetic alterations affecting the SMARCB1 gene (hSNF5/INI1), and a poor prognosis. Whether INI1 plays a role in the pathogenesis of other central nervous system tumors is uncertain. We report on cases of 2 young children with unusual intracranial nonrhabdoid neuroectodermal tumors within and around the third or fourth ventricle that are characterized by cribriform strands and trabeculae and well-defined epithelial membrane antigen-immunopositive surfaces and show INI1 protein loss. Histological and immunohistochemical features did not correspond to established tumor types, including atypical teratoid/rhabdoid tumors, medulloepithelioma, choroid plexus carcinoma, and ependymoma. Fluorescence in situ hybridization analyses failed to identify chromosomal alterations affecting the SMARCB1 locus, but sequencing revealed a homozygous 4-bp duplication in exon 4 (492duplCCTT) in one of the tumors. Both children responded well to conventional adjuvant therapy protocols and are alive and in complete remission longer than 5 years postoperatively. We suggest that cribriform neuroepithelial tumor (CRINET) is a nonrhabdoid ventricular tumor that shows loss of tumoral INI1 protein and has a relatively favorable prognosis.
AB - Atypical teratoid/rhabdoid tumors are malignant embryonal tumors characterized by the presence of rhabdoid cells, genetic alterations affecting the SMARCB1 gene (hSNF5/INI1), and a poor prognosis. Whether INI1 plays a role in the pathogenesis of other central nervous system tumors is uncertain. We report on cases of 2 young children with unusual intracranial nonrhabdoid neuroectodermal tumors within and around the third or fourth ventricle that are characterized by cribriform strands and trabeculae and well-defined epithelial membrane antigen-immunopositive surfaces and show INI1 protein loss. Histological and immunohistochemical features did not correspond to established tumor types, including atypical teratoid/rhabdoid tumors, medulloepithelioma, choroid plexus carcinoma, and ependymoma. Fluorescence in situ hybridization analyses failed to identify chromosomal alterations affecting the SMARCB1 locus, but sequencing revealed a homozygous 4-bp duplication in exon 4 (492duplCCTT) in one of the tumors. Both children responded well to conventional adjuvant therapy protocols and are alive and in complete remission longer than 5 years postoperatively. We suggest that cribriform neuroepithelial tumor (CRINET) is a nonrhabdoid ventricular tumor that shows loss of tumoral INI1 protein and has a relatively favorable prognosis.
KW - Antineoplastic Combined Chemotherapy Protocols
KW - Brain Neoplasms
KW - Child, Preschool
KW - Chromosomal Proteins, Non-Histone
KW - Combined Modality Therapy
KW - DNA-Binding Proteins
KW - Female
KW - Fourth Ventricle
KW - Humans
KW - Immunohistochemistry
KW - In Situ Hybridization, Fluorescence
KW - Infant
KW - Male
KW - Neoplasms, Neuroepithelial
KW - Neurosurgical Procedures
KW - Prognosis
KW - Radiotherapy
KW - Third Ventricle
KW - Transcription Factors
U2 - 10.1097/NEN.0b013e3181c06a51
DO - 10.1097/NEN.0b013e3181c06a51
M3 - SCORING: Journal article
C2 - 19915490
VL - 68
SP - 1249
EP - 1255
JO - J NEUROPATH EXP NEUR
JF - J NEUROPATH EXP NEUR
SN - 0022-3069
IS - 12
M1 - 12
ER -